Clinical worsening in reversible cerebral vasoconstriction syndrome

JAMA Neurol. 2014 Jan;71(1):68-73. doi: 10.1001/jamaneurol.2013.4639.


Importance: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by recurrent thunderclap headaches and evidence of vasoconstriction with subsequent resolution. The clinical course of RCVS is traditionally considered monophasic and benign. However, recurrent episodes of focal neurological symptoms have been described after initial presentation.

Objective: To define the frequency, timing, and consequences of clinical worsening in patients with diagnosis of RCVS.

Design, setting, and participants: Retrospective observational study of consecutive patients with RCVS at 2 referral institutions for neurological disease.

Main outcome and measure: Clinical worsening after diagnosis of RCVS. We defined clinical worsening as new permanent or transient neurological deficits (compared with presenting signs and symptoms) or new onset of seizures. We performed a logistic regression analysis to assess associations between patient characteristics and clinical worsening. Functional outcome was assessed at 1 to 3 months using the modified Rankin score.

Results: We identified 59 patients (median age, 47 years; interquartile range, 32-54 years) with RCVS. Twenty patients (34%) experienced clinical worsening after a median of 2.5 days (range, several hours to 14 days). Eight of the 20 patients who worsened had permanent deficits, including 4 who died. We did not find an association between age, sex, smoking, migraine, acute or chronic hypertension, peripartum state, or use of serotonergic drugs with clinical worsening. Clinical worsening was associated with radiological infarction (P = .001) and worse functional outcome (P < .004). Functional outcome was favorable (modified Rankin score 0-2) in 51 patients (86.4%).

Conclusions and relevance: Clinical worsening after diagnosis is common in patients with RCVS. Thus, RCVS is self-limited but not strictly monophasic. Most patients have a very favorable outcome, but clinical worsening may result in permanent deficits.

Publication types

  • Case Reports
  • Multicenter Study

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Disease Progression*
  • Female
  • Headache Disorders, Primary / etiology
  • Humans
  • Male
  • Middle Aged
  • Outcome Assessment, Health Care*
  • Recurrence
  • Retrospective Studies
  • Syndrome
  • Time Factors
  • Vasospasm, Intracranial / complications
  • Vasospasm, Intracranial / epidemiology*
  • Vasospasm, Intracranial / mortality
  • Young Adult